Immunoglobulin M (IgM) multiple myeloma versus Waldenström macroglobulinemia: diagnostic challenges and therapeutic options: two case reports
Open Access
- 22 June 2020
- journal article
- research article
- Published by Springer Science and Business Media LLC in Journal of Medical Case Reports
- Vol. 14 (1), 1-7
- https://doi.org/10.1186/s13256-020-02380-2
Abstract
Immunoglobulin M multiple myeloma and Waldenström macroglobulinemia are two different hematological diseases with the common finding of an immunoglobulin M monoclonal gammopathy of unknown significance. However, clinical characteristics of the two entities can overlap. In this report, we describe two cases of immunoglobulin M neoplasm with the same histological bone marrow presentation but with different clinical behavior, cytogenetics, and biological assessment. On the basis of comprehensive diagnostic workup, these patients were considered to have different diseases and treated accordingly with different approaches. Patient 1 (Caucasian man) presented with increased serum protein and immunoglobulin M (7665 mg/L) with an M-spike electrophoresis of 4600 mg/L. His bone marrow biopsy revealed a small-cell immunoglobulin M multiple myeloma. The result of testing for the MYD88 L265P mutation was negative, while fluorescence in situ hybridization analysis showed translocation t(11,14). A diagnosis of immunoglobulin M-κ multiple myeloma was made. Patient 1 was a candidate for bortezomib plus thalidomide and dexamethasone, followed by autologous stem cell transplant consolidation. Patient 2 (Caucasian man) showed an M-spike by protein electrophoresis (300 mg/L, 4.9%), with serum immunoglobulin M level of 327 mg/L. His bone marrow biopsy revealed immunoglobulin M-κ multiple myeloma. Computed tomography showed many enlarged lymph nodes and splenomegaly. Patient 2’s clinical features were suggestive of Waldenström macroglobulinemia, in contrast to the bone marrow biopsy results. The result of testing for the MYD88 L265P mutation was positive. Patient 2 was diagnosed with Waldenström macroglobulinemia and received rituximab, cyclophosphamide, and dexamethasone. A correct differential diagnosis between immunoglobulin M multiple myeloma and Waldenström macroglobulinemia is a critical point in the setting of a new immunoglobulin M monoclonal gammopathy onset. These patients should undergo a complete diagnostic workup with pathological, radiological, and serological examinations to establish the diagnosis and plan the most appropriate treatment in order to improve the prognosis.Keywords
This publication has 10 references indexed in Scilit:
- Waldenström’s macroglobulinaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-upAnnals of Oncology, 2018
- [Diagnostic approach of an IgM monoclonal gammopathy and clinical importance of gene MYD88 L265P mutation].Revue Medicale de Bruxelles, 2018
- Case Report of IgM Multiple MyelomaCancer Control, 2018
- Lenalidomide, Bortezomib, and Dexamethasone with Transplantation for MyelomaThe New England Journal of Medicine, 2017
- IgM myeloma: A multicenter retrospective study of 134 patientsAmerican Journal of Hematology, 2017
- MYD88 Mutations and Response to Ibrutinib in Waldenström's MacroglobulinemiaThe New England Journal of Medicine, 2015
- Bendamustine plus rituximab versus CHOP plus rituximab as first-line treatment for patients with indolent and mantle-cell lymphomas: an open-label, multicentre, randomised, phase 3 non-inferiority trialThe Lancet, 2013
- IgM multiple myeloma: Disease definition, prognosis, and differentiation from Waldenstrom's macroglobulinemiaAmerican Journal of Hematology, 2010
- Gene-expression profiling of Waldenström macroglobulinemia reveals a phenotype more similar to chronic lymphocytic leukemia than multiple myelomaBlood, 2006
- Translocation t(11;14)(q13;q32) is the hallmark of IgM, IgE, and nonsecretory multiple myeloma variantsBlood, 2003